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DNA분석에 기포한 표피박리성 수장족저 각피증 ( Epidermolytic palmoplantar keratoderma ) 의 산전 진단
양순하(Soon Ha Yang),노정래(Cheong Rae Roh),이제호(Je Ho Lee),정재현(Jae Hyun Chung),문종수(Zong Soo Moon),양준모(Jun Mo Yang),김승욱(Syng Wook Kim) 대한산부인과학회 1999 Obstetrics & Gynecology Science Vol.42 No.1
Objective: The purpose of this investigation was to establish the prenatal diagnosis for identifying the risk for epidermolytic palmoplantar keratoderma(EPPK) of a fetus by sequence analysis of fetal genomic DNA from chorionic villi. Methods: Chorionic villus sampling under transvaginal sonography at 12 weeks of gestation from a woman at risk for a child in a EPPK-affected family was perfomed. Polymerase chain reaction amplification of specific allele (PASA) assay was carried out for the detection of mutation(R162W in keratin 9 [K9] gene) previously identified in this family. Direct DNA sequencing analysis of K9 gene was accomplished to confirm the mutation. Results: We had found the point mutation, R162W of K9 gene, in affected family members and confirmed by PASA assay. Affected family members were shown to have PCR products reactive with both the mutant and wildtype specific primers. Because we could not find any expected products after PASA assay with the primers la(+)/KSmt(-) of the fetal DNA, we predicted that the fetus did not inherited the mutant allele and that the fetus could be unaffected. After PASA assay, we analyzed DNA sequences of two family members to confirm the mutation. A C-to-T substitution at bp 545 was detected in the father, instead the fetus did not have any mutant band at that base pair. Conclusion: The PASA assay and direct DNA sequencing analysis of K9 gene through chorionic villi sampling and extraction of genomic DNA had validity to early prenatal diagnosis whether fetus was affected in EPPK or not.
임신중 심부전 및 조기분만진통과 합병된 근긴장성 이영양증 1 예
양순하(Soon Ha Yang),노정래(Cheong Rae Roh),정재현(Jae Hyun Chung),문종수(Zong Soo Moon),문종택(Jong Taek Moon),김병준(B . Joon Kim) 대한산부인과학회 1999 Obstetrics & Gynecology Science Vol.42 No.2
Myotonic dystrophy is an autosomal-dominantly inherited neuromuscular disorder characterized by slowly progressive muscular dystrophy, muscle weakness and myotonia. The clinical features may vary from just cataracts to involvement of multiple organ systems such as various muscles, heart, lung and intestine. During pregnancy and delivery, serious maternal and obstetrical complications may occur. The myotonia is often aggravated during pregnancy and it leads to obstetrical complications such as fetal loss, preterm premature delivery, hydrops, in-utero fetal death, difficulties in fetal expulsion, postpartum hemorrhage and/or anesthetic accidents. The affected neonate may display severe hypotonia, facial displegia and respiratory distress. This report presents a woman with myotonic dystrophy complicated with congestive heart failure and preterm delivery during pregnancy.
임신오조와 동반된 Wernicke - Korsakoff 증후군 1 예
양순하(Soon Ha Yang),노정래(Cheong Rae Roh),이재성(Jae Sung Lee),문희수(Heui Soo Moon),정재현(Jae Hyun Cheung) 대한산부인과학회 1999 Obstetrics & Gynecology Science Vol.42 No.2
A 24-year-old pregnant woman started to have severe hyperemesis gravidarum at 6 weeks' gestation. Six weeks later, dizziness, ataxia, visual disturbance, diplopia and confusion were developed. On admission, she presented ophthalmoplegia, nystagmus, deaeased tendon reflex, intention tremor, ataxia, confusion and memory disturbance. She was diagnosed to Wernicke-Korsakoff syndrome and 100mg of thiamine was administered intravenously daily till 28 weeks gestation and then intramuscularly. Her ocular symptns, together with neurological signs, were gradually improved. Memory disturbance slightly improved but remained at the time of delivery. The case will be presented in more details with a brief revie of literatures.
정상 한국인 임신부에서 임신주수에 따른 자궁경부길이의 변화양상
양순하 ( Soon Ha Yang ),최성진 ( Seong Jin Choi ) 대한산부인과학회 2002 Obstetrics & Gynecology Science Vol.45 No.11
Objective: The aims of this study are to evaluate the longitudinal change in the cervix throughout pregnancy by transvaginal ultrasound and to establish a normogram of the cervical length in Korean pregnant women. Methods: A prospective observational stud
자궁경부의 개대가 진행된 자궁경부무력증에서 응급 자궁경부봉축술의 유용성
양순하 ( Soon Ha Yang ),김지운 ( Ji Un Kim ),최성진 ( Seong Jin Choi ),서용수 ( Yong Soo Seo ),김영아 ( Young Ah Kim ),김종화 ( Jong Hwa Kim ) 대한산부인과학회 2003 Obstetrics & Gynecology Science Vol.46 No.3
Objective : To investigate the clinical significance of emergency cerclage on prologation of pregnancy and perinatal outcome in mid-trimester cervical incompetence with advanced cervical dilatation. Methods : We analyzed the pregnancy outcome retrospectiv
만삭산모에서 유도분만의 성공예측지표로서 질식초음파를 이용한 자궁경부 관찰의 유용성
양순하(Soon Ha Yang),오정미(Jung Mi Oh),노정래(Cheong Rae Roh),정재현(Jae Hyun Chung) 대한산부인과학회 1998 Obstetrics & Gynecology Science Vol.41 No.11
Objectives: The purposes of this study were to determine the usefulness of transvaginal ultrasonographic assessment of the uterine cervix and to compare the diagnostic performance of ultrasonographic and digital examination of the cervix in predicting a successful induction of labor. Study design: One hundred-one singleton term pregnancies without ruptured membranes admitted for the labor induction were included in this study. Digital examination and transvaginal ultrasonography of the uterine cervix were performed at the time of admission. Cervical parameters evaluated included cervical length, presence of funneling, funnel length, and funnel width. Labor induction was underwent by prostaglandin E2 (PGE2) vaginal suppository and/or pitocin intravenous infusion. Outcome variable was a successful labor induction within 48 hours after beginning of the induction. Results: The prevalence of induction failure was 10.9% (11/101). Receiver-operator characteristic (ROC) curve and multiple logistic regression analysis indicated a significant relationship between the successful induction of labor and cervical length <3.1 cm. The diagnositic indices of endocervical length was superior to those of Bishop's cervical score in predicting a successful induction of labor. In patients with cervical length <3.1 cm, the labor was induced successfully with fewer tablets of PGE2, less use of pitocin infusion, and shorter induction-delivery interval. Conclusion: Transvaginal ultrasonographical examination of the uterine cervix is more accurate than digital examination of the cervix in the prediction of a successful induction of labor in term gestation.
만삭전 조기양막파열에서 분만 잠복기의 예측지표로서 입원시 측정한 양수지수의 유용성
양순하(Soon Ha Yang),박선혜(Seon Hye Park),오성희(Sung Hee Oh),황종대(Jong Dae Whang),노정래(Cheong Rae Roh) 대한산부인과학회 1999 Obstetrics & Gynecology Science Vol.42 No.12
Objectives: The purpose of this study was to determine the clinical significance of residual amniotic fluid volume, as measured by the amniotic fluid index(AFI), on the prediction of latency period and perinatal outcomes in patients with preterm premature rupture of the membranes(PPROM). Methods: Study population consisted of 103 singleton pregnancies with PPROM between 24 and 34 weeks' gestation. Amniotic fluid index was determined using transabdominal ultrasound at admission. Latency period was defined as time interval in hours between admission and delivery. All medical records of mothers and neonates were reviewed. Spearman's rho rank correlation, receiver-operator characteristic(ROC) curve analysis, survival analysis and Cox's proportional hazard model were used for statistical analysis. Results: There was a statistically significant correlation between latency period and AFI at admission. ROC curve analysis showed that AFI at admission had a significant predictive value in the prediction of latency period < 48 hours. Survival analysis demonstrated that AFI < 5.0 was strongly associated with the likelihood of shorter latency period, Cox's proportional hazard model indicated that AFI < 5.0 was a significant independent predictor for the occurrence of latency period < 48 hours even after adjustment of other independent variables. The patients with AFI < 5.0 had a higher rate of cesarean section due to fetal distress than those with AFI > 5.0 but the incidence of chorioamnionitis was not significantly different between two groups. Comparing the perinatal mortality and neonatal morbidity including infectious morbidity between the neonates born to mothers with AFI < 5.0 and those with AFI > 5.0, there was no significance after adjustment of gestational age at birth. Conclusion: AFI at admission has a significant correlation with latency period and predictive value in prediction of latency period in the patients with PPROM. AFI < 5.0 is a independent predictor for the shorter latency period.
만삭 자궁근세포에서 염증성 사이토카인에 의한 nuclear factor-kappa B 활성화 양상의 진통 유무에 따른 차이 비교
양순하 ( Soon Ha Yang ),신희철 ( Hee Chul Syn ) 대한산부인과학회 2005 Obstetrics & Gynecology Science Vol.48 No.3
Objective: The objectives of this study were to determine whether NF-κB-mediated pro-inflammatory cytokines signaling leads to COX-2 expression and PGE2 synthesis in human myometrial cells at term, and to compare cytokines-induced NF-κB activity and subse